Incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS).

Erythema

Redness of the skin, caused by hyperemia of the capillaries in the lower layers of the skin.


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PMID (PMCID)
1628974
 FEMALE Infant, Newborn
[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
Schaller J, Kock M, Goos M.
Hautarzt. 1992;43(6):383-5.
The case report of a 2-week-old girl suffering from incontinentia pigmenti (Bloch-Sulzberger Syndrome) with disseminated urticarial erythemas, papules and vesicles at the onset is presented.
1628974
 FEMALE Infant, Newborn
[Onset of disseminated Bloch-Sulzberger incontinentia pigmenti].
Schaller J, Kock M, Goos M.
Hautarzt. 1992;43(6):383-5.
The case report of a 2-week-old girl suffering from incontinentia pigmenti (Bloch-Sulzberger Syndrome) with disseminated urticarial erythemas, papules and vesicles at the onset is presented.
1200665
 FEMALE Child
Incontinentia pigmenti and defective neutrophil chemotaxis.
Dahl MV, Matula G, Leonards R, Tuffanelli DL.
Arch Dermatol. 1975;111(12):1603-5.
A child with incontinentia pigmenti and chronic erythema multiforme had recurrent bacterial infections.